Increased prevalence of iron-overload associated endocrinopathy in thalassaemia versus sickle-cell disease

Ellen B. Fung, Paul R. Harmatz, Phillip Lee, Meredith Milet, Rita Bellevue, Michael R. Jeng, Karen A. Kalinyak, Mark Hudes, Suruchi Bhatia, Elliott P. Vichinsky

Research output: Contribution to journalArticle

118 Citations (Scopus)

Abstract

Iron-overload associated endocrinopathy is the most frequently reported complication of chronic transfusion therapy in patients with thalassaemia (Thal). This study compared iron-overloaded subjects with Thal (n = 142; 54%M; age 25·8 ± 8·1 years) and transfused sickle-cell disease (Tx-SCD; n = 199; 43%M, 24·9 ± 13·2 years) to non-transfused SCD subjects (non-Tx-SCD; n = 64, 50%M, 25·3 ± 11·3 years), to explore whether the underlying haemoglobinopathy influences the development of endocrinopathy. Subjects were recruited from 31 centres in the USA, Canada and the UK. Subjects with Thal had more evidence of diabetes (13% vs. 2%, P < 0·001), hypogonadism (40% vs. 4%, P < 0·001), hypothyroidism (10% vs. 2%, P = <0·001) and growth failure (33% vs. 7%, P < 0·001), versus Tx-SCD. Fifty-six per cent of Thal had more than one endocrinopathy compared with only 13% of Tx-SCD (P < 0·001). In contrast, Tx-SCD was not different from non-Tx-SCD. Multivariate analysis indicated that endocrinopathy was more likely in Thal than SCD [Odds Ratio (OR) = 9·4, P < 0·001], with duration of chronic transfusion a significant predictor (OR = 1·4 per 10 years of transfusion, P = 0·04). Despite iron overload, endocrinopathy was not increased in Tx-SCD versus non-Tx-SCD, suggesting that the underlying disease may modulate iron-related endocrine injury. However, because transfusion duration remained a significant predictor of endocrinopathy, these data should be confirmed in SCD subjects that have been chronically transfused for longer periods of time.

Original languageEnglish (US)
Pages (from-to)574-582
Number of pages9
JournalBritish Journal of Haematology
Volume135
Issue number4
DOIs
StatePublished - Nov 2006
Externally publishedYes

Fingerprint

Iron Overload
Thalassemia
Sickle Cell Anemia
Iron
Odds Ratio
Hemoglobinopathies
Hypogonadism
Hypothyroidism
Canada
Multivariate Analysis
Wounds and Injuries
Growth

Keywords

  • Endocrinopathy
  • Iron overload
  • Sickle-cell disease
  • Thalassaemia
  • Transfusion therapy

ASJC Scopus subject areas

  • Hematology

Cite this

Increased prevalence of iron-overload associated endocrinopathy in thalassaemia versus sickle-cell disease. / Fung, Ellen B.; Harmatz, Paul R.; Lee, Phillip; Milet, Meredith; Bellevue, Rita; Jeng, Michael R.; Kalinyak, Karen A.; Hudes, Mark; Bhatia, Suruchi; Vichinsky, Elliott P.

In: British Journal of Haematology, Vol. 135, No. 4, 11.2006, p. 574-582.

Research output: Contribution to journalArticle

Fung, EB, Harmatz, PR, Lee, P, Milet, M, Bellevue, R, Jeng, MR, Kalinyak, KA, Hudes, M, Bhatia, S & Vichinsky, EP 2006, 'Increased prevalence of iron-overload associated endocrinopathy in thalassaemia versus sickle-cell disease', British Journal of Haematology, vol. 135, no. 4, pp. 574-582. https://doi.org/10.1111/j.1365-2141.2006.06332.x
Fung, Ellen B. ; Harmatz, Paul R. ; Lee, Phillip ; Milet, Meredith ; Bellevue, Rita ; Jeng, Michael R. ; Kalinyak, Karen A. ; Hudes, Mark ; Bhatia, Suruchi ; Vichinsky, Elliott P. / Increased prevalence of iron-overload associated endocrinopathy in thalassaemia versus sickle-cell disease. In: British Journal of Haematology. 2006 ; Vol. 135, No. 4. pp. 574-582.
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abstract = "Iron-overload associated endocrinopathy is the most frequently reported complication of chronic transfusion therapy in patients with thalassaemia (Thal). This study compared iron-overloaded subjects with Thal (n = 142; 54{\%}M; age 25·8 ± 8·1 years) and transfused sickle-cell disease (Tx-SCD; n = 199; 43{\%}M, 24·9 ± 13·2 years) to non-transfused SCD subjects (non-Tx-SCD; n = 64, 50{\%}M, 25·3 ± 11·3 years), to explore whether the underlying haemoglobinopathy influences the development of endocrinopathy. Subjects were recruited from 31 centres in the USA, Canada and the UK. Subjects with Thal had more evidence of diabetes (13{\%} vs. 2{\%}, P < 0·001), hypogonadism (40{\%} vs. 4{\%}, P < 0·001), hypothyroidism (10{\%} vs. 2{\%}, P = <0·001) and growth failure (33{\%} vs. 7{\%}, P < 0·001), versus Tx-SCD. Fifty-six per cent of Thal had more than one endocrinopathy compared with only 13{\%} of Tx-SCD (P < 0·001). In contrast, Tx-SCD was not different from non-Tx-SCD. Multivariate analysis indicated that endocrinopathy was more likely in Thal than SCD [Odds Ratio (OR) = 9·4, P < 0·001], with duration of chronic transfusion a significant predictor (OR = 1·4 per 10 years of transfusion, P = 0·04). Despite iron overload, endocrinopathy was not increased in Tx-SCD versus non-Tx-SCD, suggesting that the underlying disease may modulate iron-related endocrine injury. However, because transfusion duration remained a significant predictor of endocrinopathy, these data should be confirmed in SCD subjects that have been chronically transfused for longer periods of time.",
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